Purpose To assess the challenges and management of carotid body tumors (CBTs). Adequate planning by the surgeon can greatly reduce adverse events of such rare and difficult tumors. Background CBTs are rare. Despite over a century of describing surgery, controversy remains surrounding details of their treatment. Most complications are considered technically preventable. Patients and methods This prospective study included 13 cases diagnosed as CBT. Patients were subjected to clinical evaluation, carotid duplex ultrasound, computed tomography angiography scan, and sometimes MR angiography. All patients underwent CBT resection and when needed vascular reconstruction. Patients were classified into three groups depending on Shamblin classification. Results Mean operative time was longer in patients of Shamblin class 3 (5.4±0.77 h; range, 4.5–6h; P<0.001). Regarding vascular reconstruction, all patients of Shamblin class 3 were reconstructed by interposition vein graft and two patients of Shamblin class 1 were reconstructed through primary repair, but patients of Shamblin class 2 were reconstructed by all means. In postoperative follow-up complications, bleeding was more significantly in Shamblin class 3 (P=0.032). Cranial nerve deficit was observed in four (33.3%) patients, and all of them were transient. The mean size of excised tumor was 5.17±1.4 cm, with range of 3–7.5 cm. Conclusion Early surgical excision by a vascular surgeon is the only proven cure for CBTs, because they are small and easy to remove. However, if neglected, there will be many challenges, as most tumors can become locally invasive. Meticulous periadventitial (and sometimes subadventitial) dissection can greatly reduce the rates of complications.